难治性乳糜泻。

Refractory Celiac Disease.

机构信息

Paris Descartes University, 15 rue de l'Ecole de Médecine, Paris 75006, France; Gastroenterology Department, Hôpital Européen Georges Pompidou, APHP, 20 rue Leblanc, Paris 75015, France; UMR1163 Institute Imagine, 24 Boulevard du Montparnasse, Paris 75015, France.

出版信息

Gastroenterol Clin North Am. 2019 Mar;48(1):137-144. doi: 10.1016/j.gtc.2018.09.010. Epub 2018 Dec 13.

DOI:10.1016/j.gtc.2018.09.010

PMID:30711206

Abstract

Refractory celiac disease (RCD) refers to persistence of malnutrition and intestinal villous atrophy for more than 1 to 2 years despite strict gluten-free diet in patients with celiac disease. Diagnosis remains difficult and impacts treatment and follow-up. RCD has been subdivided into 2 subgroups according to the normal (RCDI) or abnormal phenotype of intraepithelial lymphocytes (IELs) (RCDII). RCDII is considered as a low-grade intraepithelial lymphoma and has a poor prognosis due to gastrointestinal and extraintestinal dissemination of the abnormal IELs, and high risk of overt lymphoma.

摘要

难治性乳糜泻（RCD）是指乳糜泻患者在严格的无麸质饮食下，仍持续存在 1 至 2 年以上的营养不良和肠绒毛萎缩。其诊断仍然具有挑战性，影响治疗和随访。RCD 根据肠上皮内淋巴细胞（IELs）的正常（RCDI）或异常表型（RCDII）分为 2 个亚组。RCDII 被认为是一种低度上皮内淋巴瘤，由于异常 IEL 的胃肠道和肠外播散以及明显淋巴瘤的高风险，预后较差。